Intest Res.  2022 Apr;20(2):224-230. 10.5217/ir.2021.00042.

Association of young age and male sex with primary sclerosing cholangitis in Taiwanese patients with inflammatory bowel disease

• Weng MT
• Shih IL
• Tung CC
• Leong YL
• Shieh MJ
• Wang CY
• Wong JM
• Ni YH
• Wei SC

• KMID: 2529568
• DOI: http://doi.org/10.5217/ir.2021.00042

Abstract

Background/Aims
Primary sclerosing cholangitis (PSC) is associated with inflammatory bowel disease (IBD). We aimed to evaluate the prevalence, clinical manifestation, and outcomes of PSC in Taiwanese patients with IBD. Methods: This retrospective study enrolled patients with IBD admitted from January 1, 1996, to December 31, 2018, to National Taiwan University Hospital. A case-matched analysis was performed comparing patients with IBD with and without PSC according to age, sex, and time of admission, with ratios of 1:4 and 1:2 in the adult and pediatric groups, respectively.
Results
In total, 763 patients with IBD were enrolled, 12 of whom were also diagnosed with PSC (1.57%). All these patients had ulcerative colitis (UC). A greater incidence of IBD with PSC was observed in younger patients than in older patients. Male sex was a risk factor for PSC in pediatric patients with IBD (P=0.015); 75% of these patients were diagnosed with PSC along with or after the diagnosis of UC. There was no significant difference in colitis extent and severity between the groups; however, a higher proportion of rectal sparing was observed in patients with PSC (P=0.001). There was no significant difference in cancer development between the groups (P=0.679). Conclusions: A 1.57% prevalence of PSC was observed in Taiwanese patients with IBD. The majority of patients with IBD and PSC were men and were diagnosed at a younger age. Hence, routine evaluation of biliary enzymes and liver imaging is recommended in young male patients with IBD.

Keyword

Primary sclerosing cholangitis; Inflammatory bowel disease; Ulcerative colitis

Reference

1. Boonstra K, Beuers U, Ponsioen CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review. J Hepatol. 2012; 56:1181–1188.

2. Boonstra K, Weersma RK, van Erpecum KJ, et al. Populationbased epidemiology, malignancy risk, and outcome of primary sclerosing cholangitis. Hepatology. 2013; 58:2045–2055.

3. Karlsen TH, Folseraas T, Thorburn D, Vesterhus M. Primary sclerosing cholangitis: a comprehensive review. J Hepatol. 2017; 67:1298–1323.

4. de Vries AB, Janse M, Blokzijl H, Weersma RK. Distinctive inflammatory bowel disease phenotype in primary sclerosing cholangitis. World J Gastroenterol. 2015; 21:1956–1971.

5. Yang BR, Choi NK, Kim MS, et al. Prevalence of extraintestinal manifestations in Korean inflammatory bowel disease patients. PLoS One. 2018; 13:e0200363.

6. Vavricka SR, Rogler G, Gantenbein C, et al. Chronological order of appearance of extraintestinal manifestations relative to the time of IBD diagnosis in the Swiss Inflammatory Bowel Disease Cohort. Inflamm Bowel Dis. 2015; 21:1794–1800.

7. Folseraas T, Boberg KM. Cancer risk and surveillance in primary sclerosing cholangitis. Clin Liver Dis. 2016; 20:79–98.

8. Truelove SC, Witts LJ. Cortisone in ulcerative colitis; preliminary report on a therapeutic trial. Br Med J. 1954; 2:375–378.

9. Núñez F P, Quera P R, Gomollón F. Primary sclerosing cholangitis and inflammatory bowel disease: intestine-liver interrelation. Gastroenterol Hepatol. 2019; 42:316–325.

10. Yen HH, Weng MT, Tung CC, et al. Epidemiological trend in inflammatory bowel disease in Taiwan from 2001 to 2015: a nationwide populationbased study. Intest Res. 2019; 17:54–62.

11. Wei SC, Shieh MJ, Chang MC, Chang YT, Wang CY, Wong JM. Long-term follow-up of ulcerative colitis in Taiwan. J Chin Med Assoc. 2012; 75:151–155.

12. Dignass A, Eliakim R, Magro F, et al. Second European evidence-based consensus on the diagnosis and management of ulcerative colitis part 1: definitions and diagnosis. J Crohns Colitis. 2012; 6:965–990.

13. Lin WC, Weng MT, Tung CC, et al. Trends and risk factors of mortality analysis in patients with inflammatory bowel disease: a Taiwanese nationwide population-based study. J Transl Med. 2019; 17:414.

14. Singh B, Kedia S, Konijeti G, et al. Extraintestinal manifestations of inflammatory bowel disease and intestinal tuberculosis: frequency and relation with disease phenotype. Indian J Gastroenterol. 2015; 34:43–50.

15. Fagundes ED, Ferreira AR, Hosken CC, Queiroz TC. Primary sclerosing cholangitis in children and adolescents. Arq Gastroenterol. 2017; 54:286–291.

16. Fousekis FS, Theopistos VI, Mitselos IV, et al. Specific features of patients with inflammatory bowel disease and primary sclerosing cholangitis. J Clin Med Res. 2019; 11:81–88.

17. Bajer L, Wohl P, Drastich P. PSC-IBD: specific phenotype of inflammatory bowel disease associated with primary sclerosing cholangitis. Vnitr Lek. 2018; 64:659–664.

18. Chou JW, Lai HC, Chang CH, Cheng KS, Feng CL, Chen TW. Epidemiology and clinical outcomes of inflammatory bowel disease: a hospital-based study in central Taiwan. Gastroenterol Res Pract. 2019; 2019:4175923.

19. Park SH, Yang SK, Park SK, et al. Atypical distribution of inflammation in newly diagnosed ulcerative colitis is not rare. Can J Gastroenterol Hepatol. 2014; 28:125–130.

20. Horio Y, Uchino M, Bando T, et al. Rectal-sparing type of ulcerative colitis predicts lack of response to pharmacotherapies. BMC Surg. 2017; 17:59.

21. Ricciuto A, Kamath BM, Griffiths AM. The IBD and PSC phenotypes of PSC-IBD. Curr Gastroenterol Rep. 2018; 20:16.

22. Palmela C, Peerani F, Castaneda D, Torres J, Itzkowitz SH. Inflammatory bowel disease and primary sclerosing cholangitis: a review of the phenotype and associated specific features. Gut Liver. 2018; 12:17–29.

23. Jess T, Rungoe C, Peyrin-Biroulet L. Risk of colorectal cancer in patients with ulcerative colitis: a meta-analysis of population-based cohort studies. Clin Gastroenterol Hepatol. 2012; 10:639–645.

24. Zheng HH, Jiang XL. Increased risk of colorectal neoplasia in patients with primary sclerosing cholangitis and inflammatory bowel disease: a meta-analysis of 16 observational studies. Eur J Gastroenterol Hepatol. 2016; 28:383–390.

25. Weismüller TJ, Trivedi PJ, Bergquist A, et al. Patient age, sex, and inflammatory bowel disease phenotype associate with course of primary sclerosing cholangitis. Gastroenterology. 2017; 152:1975–1984.